Amyotrophic lateral sclerosis (ALS) is a nervous system disorder that steadily worsens over time. It affects nerves in the brain and spine that are responsible for muscle movement. The nerves gradually die which can lead to almost total paralysis, including being unable to breathe. ALS is fatal, often due to respiratory failure.
The cause of ALS is unknown. Genes may play a role.
The symptoms of ALS are caused by the death of the nerves. If the nerves can not send signals to the muscle they are not able to work. Overtime the muscles become weak and shrink from disuse.
Factors that may increase your chance of ALS include:
Military veterans have twice the risk of ALS as the general population. It is not clear why.
The first signs of ALS are subtle and can be different from person to person. They may include:
The speed of the progression can be very different between people. As the disease progresses it can lead to:
You will be asked about your symptoms and medical history. A physical exam will be done. There are no tests that can specifically diagnose ALS. Instead, tests will be done to rule out other medical conditions.
Imaging tests to look for changes in the brain or spine may include:
Other tests may include:
Your muscles and nerves may also be checked. This can be done with electromyogram (EMG)/nerve conduction velocities (NCV).
Your thinking and processing skills may also be checked.
There is currently no cure for ALS. A combination of treatments may help to reduce or manage symptoms. Treatment options include:
The drug riluzole and edaravone have been approved for ALS. The drugs may slightly improve functioning, but it does not stop the disease from progressing.
Medications may include:
Supportive care may be needed as ALS progresses, including:
National Institute of Neurological Disorders and Stroke
Amyotrophic Lateral Sclerosis Society of British Columbia
Aggarwal, SP, Zinman L, Simpson E, et al. Clinical trial testing lithium in ALS terminates early for futility. Lancet Neurol. 2010;9(5):481-488.
Amyotrophic lateral sclerosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS. Updated July 25, 2016. Accessed September 30, 2016.
Amyotrophic lateral sclerosis (ALS) fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm. Updated February 1, 2016. Accessed February 12, 2016.
NeuRx Diaphragm Pacing System. Food and Drug Administration website. Available at: http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm. Updated September 6, 2013. Accessed February 12, 2016.
Sathasivam S. Managing patients with amyotrophic lateral sclerosis. Eur J Intern Med. 2009;20(4):355-358.
Walling AD. Amyotrophic lateral sclerosis: Lou Gehrig's disease. Am Fam Physician. 1999;59(6):1489-1496.
4/17/2008 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Fornai F, Longone P, Cafaro L, et al. Lithium delays progression of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2008;105(6):2052-2057.
1/14/2011 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Mateen FJ, Carone M, Sorenson EJ. Patients who survive 5 years or more with ALS in Olmsted County, 1925-2004. J Neurol Neurosurg Psychiatry. 2010;81(10):1144-1146.
9/3/2014 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Wippold FJ, Cornelius RS, Broderick DF, et al. American College of Radiology (ACR) Appropriateness Criteria for dementia and movement disorders. Available at: http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/DementiaAndMovementDisorders.pdf. Updated 2014. Accessed February 12, 2016.
9/26/2017 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: US Food and Drug Administration. FDA approves drug to treat ALS. Available at: https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm557102.htm. Published May 5, 2017. Accessed September 26, 2017.
Last reviewed March 2017 by EBSCO Medical Review Board Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.